Neurocutaneous melanosis in association with the Dandy-Walker complex

Pediatr Dermatol. 1992 Mar;9(1):37-43. doi: 10.1111/j.1525-1470.1992.tb00323.x.


An infant had a giant congenital nevus, neurocutaneous melanosis (NCM), and a Dandy-Walker malformation of the brain. The diagnosis of NCM was suspected at 6 weeks of age when macrocephaly was noted, resulting in the discovery of hydrocephalus and a Dandy-Walker malformation. Serial magnetic resonance imaging scans demonstrated so-called T1 shortening in the pia or subarachnoid spaces surrounding the cerebellar vermis and in the temporal lobes anterior to the temporal horns. Eventually, a biopsy-proved melanoma developed in the anterior temporal lobe, in an area previously noted to have T1 shortening. Since meningeal cells have been shown experimentally to play a critical role in cerebellar development, we hypothesize that the association of NCM with a Dandy-Walker malformation may be due to meningeal melanosis disrupting the normal development of the cerebellum and fourth ventricle.

Publication types

  • Case Reports

MeSH terms

  • Brain Neoplasms / complications
  • Brain Neoplasms / diagnosis*
  • Dandy-Walker Syndrome / complications
  • Dandy-Walker Syndrome / diagnosis*
  • Humans
  • Infant
  • Magnetic Resonance Imaging
  • Male
  • Melanoma / complications
  • Melanoma / diagnosis*
  • Melanosis / complications
  • Melanosis / diagnosis*
  • Nevus, Pigmented / complications
  • Nevus, Pigmented / congenital
  • Nevus, Pigmented / diagnosis*
  • Skin Neoplasms / complications
  • Skin Neoplasms / congenital
  • Skin Neoplasms / diagnosis*
  • Temporal Lobe*